地方性克汀病锥体外系神经细胞的形态定量研究

本文对4例地方性克汀病尸检材料的锥体外系神经细胞进行病理学检查。对其额叶皮质的第五层大锥体细胞、豆状核、黑质、齿状核以及下橄榄核的锥体细胞做了体视学的定量分析。结果锥体外系各部锥体细胞都存在不同程度的交性坏死、其数密度和体积密度均明显减少。证明地克病人锥体外路传导障碍,从而导致运动功能障碍。     

大鼠海马神经元及其线粒体增龄性改变的形态计量分析

目的探讨大鼠海马神经元及其线粒体形态结构增龄性改变的程度及性质。方法应用组织化学及电镜技术，通过计算机图像分析系统对海马神经元及其线粒体结构进行形态计量分析。结果海马ＣＡ１和ＣＡ３区神经元随增龄出现细胞皱缩，ＣＡ３区神经元数密度在老年组较青年组显著减少（Ｐ＜０．０５），ＣＡ１区神经元数密度各月龄组之间差异无显著性（Ｐ＞０．０５）。ＣＡ３区神经元胞体内线粒体体密度、数密度、比表面及嵴膜密度随增龄而减少，线粒体平均体积及平均截面积随增龄而增大。结论海马神经元及其线粒体形态结构随增龄发生显著性改变，这些形态结构的改变可能是大鼠海马老化的指征。     

地方性克汀病神经电生理学损伤初探

本文报道87例地方性克汀病人(粘肿型46例、神经型19例、混合型22例)的神经电生理学检查结果。除尺运动神经外.各型(?)者的被检外周神经传导速度多数正常;除腓肠感觉神经外,其它各被检外周神经的动作电位幅度均明显高于对照组(P<0.001)。听觉传导路在耳蜗;躯体感觉神经系统在臂丛;以及楔束核及(?)脑等部位皆有不同程度的损伤。就共整个神经电生理学损伤而言,尤以混合型为严重和广泛,而粘肿型也存在着损伤。三型的脊髓中枢感觉传导路似未受到严重损害。     

褪黑素对老年大鼠弓状核神经元影响的光镜、电镜定量研究

目的 探讨褪黑素延缓神经元衰老的作用。方法 ４０只老年ＳＤ大鼠随机分为老年组和褪黑素组,２０只成年ＳＤ大鼠为对照组,采用光镜、电镜技术和体视学方法对褪黑素延缓老年大鼠弓状核神经元衰老作用进行了形态定量研究。结果 ①三组间弓状核神经元的面积分数、面数密度、胞体和胺核的等效直径无显著变化（Ｐ〉０．０５）;②老年组弓状核暗神经元内线粒体的体积分数和面数密度明显增加（Ｐ〈０．０５）,粗面内质网、高尔基复合     

吡咯喹啉醌对培养大鼠皮层神经元的促生长作用

目的 探讨吡咯喹啉醌（PQQ）对培养鼠皮层神经元的促生长作用。方法 原代培养新生大鼠皮层神经元，观察PQQ对皮质神经元突起、胞体的生长和存活率的影响，测定MTT代谢率，免疫组化法观察c-fos蛋白的表达。结果 PQQ处理后，皮层神经元突起的长度和胞体的长径显著大于对照组，细胞的存活率增加，尼氏小体的光密度值显著高于对照组，MTT代谢率增加，c-fos的表达显著高于对照组。结论 取Ⅺ能促进培养的鼠皮层神经元的生长，增加其存活率，延长存活时间。     

氟中毒大鼠中缝背核5—羟色胺能神经元的免疫细胞化学研究

以饮用高氟水的方法制造了雄性大鼠慢性氟中毒模型。对脑干中缝背核５－羟色胺（５－ＨＴ）能神经元进行了免疫细胞化学定性、定量研究，结果显示，慢性氟中毒大鼠中缝背核５－ＨＴ能神经元略少，部分胞体缩小或肿大。胞质内５－ＨＴ阳性反应颗粒减少且界限不清。显微图像分析仪检测上述神经元内５－ＨＴ阳性反应颗粒明显减少。本研究揭示，高氟摄入对中枢５－ＨＴ能神经元有直接损害，对５－ＨＴ的产生有抑制作用。     

NMDA受体NR2B亚型基因对海马成年新生神经元形态发生的影响

目的研究NMDA受体NR2B亚型基因对海马成年新生颗粒细胞形态发生的影响。方法通过Cre-loxp重组酶系统构建NMDA受体NR2B亚型基因单细胞敲除模型。运用Neurolucida软件系统对野生型、NR2B基因敲除成年新生神经元进行形态重建,观察树突长度、树突复杂度以及棘突的变化。结果 NR2B基因敲除成年新生神经元外形与WT神经元相似,树突长度接近,棘突减少。Sholl分析显示在以胞体为中心,距胞体不同距离的树突亚区域内,树突复杂性(树突交叉)减低,与此同时棘突分布密度减低(P<0.05)。结论海马成年新生颗粒神经元NR2B基因敲除对新生细胞树突长度影响甚微,但降低了树突复杂性,减少棘突形成,从而导致了功能性整合入海马特定神经信息网络的障碍。     

VE对慢性镉中毒小鼠黑质神经元的保护作用

目的 研究维生素E(VE)对慢性镉中毒小鼠黑质神经元的保护作用。方法 小鼠染镉同时给予大量VE，通过光镜及透射电镜观察小鼠黑质神经元的细微结构，并用细胞形态计量方法对黑质区酪氨酸羟化酶(TH)阳性反应神经元进行定量分析。结果 VE加镉组小鼠中脑黑质区结构接近正常对照组，与镉中毒组比较有明显不同，黑质神经元胞核体密度、数密度等形态学参数与镉中毒组比较也差异有显著意义(P＜0．005)。结论 抗氧化剂VE对慢性镉中毒组小鼠黑质神经元有显著的保护作用。     

β-淀粉样蛋白31～35对体外培养的胚胎大鼠海马神经元的影响

对SD大鼠海马神经元行原代培养，运用MTT比色法和细胞形态学分析法，比较不同浓度的β-淀粉样蛋白（βA）31～35对神经元存活及形态的影响。结果浓度≥10μmol／L的“老化”βA31～35对体外培养的海马神经元有毒性作用，可降低细胞存活，抑制神经元胞体和突起的发育，且呈剂量依赖关系。比βA25-35序列更小的βA31-35片段对体外培养的海马神经元有神经毒性作用，可引起神经突起损伤和神经元胞体的形态学变化，这种作用与βA的浓度和聚集状态有密切关系。     

地方性克汀病人脑诱发电位及耳蜗电图研究

研究36例地方性克汀病患者的多形式脑诱发电位发现,全部病人的BAEPS异常,其中15例进行ECochG检查,表现为电平坦或N_1后延。33例的SSEPS检查,9例异常,异常率为27.27%。34例VEPS检查,8例异常,异常率为25.5%。研究结果表明:地方性克汀病人的听觉损害部位在耳蜗内,而不在蜗后听神经及脑干内的传导束上。碘和甲状腺激素缺乏引起的脑皮层萎缩及视觉传导通路某部位损害可能是SSEPS、VEPS异常的病理基础。     

地方性克汀病患者的甲状腺功能和脑组织发育

作者应用SPECT(单光子发射计算机断层扫描)超声脑血流图、EEG(脑电图)、CT(计算机断层X线扫描)、MRI(磁共振显像)和RIA(放射免疫分析法)等手段,对9例以神经系统损伤为主要表现的地方性克汀病患者进行了甲状腺、颅脑的功能和显像学研究。结果表明,患者的甲状腺和颅脑均有异常改变。听力学检查提示,患者听力障碍的病理学基础主要在听觉中枢。实践表明,上述病理性损伤用补充碘质的方法已无法使其恢复正常。     

地克病中枢神经系统和内分泌腺病理形态学研究

本文报道两例神经型地克病尸检材料,阐述了对中枢神经系统和内分泌腺的病理形态学研究结果。首次描述地克病脑边缘系统病变的广泛性。指出脑各部形态变化,特别是边缘系统的形态变化,可能是脑发育和代谢障碍的结果。这种不可逆性变化,导致受中枢神经系统控制和调节的脑垂体及其靶器官的发育、代谢和功能障碍及形态变化。这正是不同类型克汀病患者临床表现的基础。     

地方性克汀病患者子代智力甲状腺功能和生长发育

对地克病高发区居民采取补碘措施前后出生的64名地克病患者子代进行了调查。结果:检出克汀病14人(21.9%)、亚克汀病38人(59.4%)、激素性甲低9人(14.1%)。患者子女平均智商、身高、体重都低于正常人子女,甲功低下检出率高于正常人子女。两组上述各项指标的差异有显著性意义;但补碘前与补碘后出生的患者子女间基本一致。     

阿克苏部分地区地方性克汀病的遗传度调查

本文报告1973～1985年新疆阿克苏地区的地方性克汀病遗传度为58.76％,地方性克汀病的群体患病率为2.37％,其亲属患病率为7.94％,一级亲属患病率为10.03％,二级亲属患病率为5.10％,三级亲属患病率为7.64％,均与群体患病率有显著性差异(P<0.01)。     

新疆亚临床型克汀病流行病学调查报告

按照新疆ＩＤＤ流行与环境地理特征，沿山前丘陵河浴至河流下游冲积平原，对南疆喀什、巴州及北疆达板城等六个地区约３２７８名７－１４岁学龄儿童进行了全面系统的亚临床克汀病流调及临床检查。结果显示：南疆地区亚克汀 病流行强度为１４．５％，北、疆地区为５．２％，全疆平为９．１４％。     

Selenium deficiency as a possible factor in the pathogenesis of myxoedematous endemic cretinism

Myxoedematous endemic cretinism is prevalent in African goitre endemies. It has been related to a thyroid 'exhaustion' atrophy occurring near birth. It is proposed that this might result from the low resistance of a fragile tissue to enhanced H2O2 generation under intense thyroid stimulation by thyrotropin. In support of this hypothesis, low selenium and glutathione peroxidase serum levels have been found in the African endemic area of the Idjwi Island (Kivu, Zaire). Serum selenium and plasma glutathione peroxidase were lower in the area of high endemicity of goitre and cretinism (Northern part of the Island). However, only the former difference is statistically significant. These data thus suggest a role of oligoelements and oxygen toxicity in the pathogenesis of endemic cretinism.     

Studies on hearing loss in a community with endemic cretinism in Central Java, Indonesia.

An audiometric study was carried out in a community of 642 subjects severly affected by endemic goitre and cretinism. Hearing loss was measured in 34 out of 41 subjects diagnosed as cretins, 92 normal subjects aged 5-20 years from the same community and 54 subjects (also of 5-20 years) living in a nearby control area without endemic goitre. The excess number of hearing defects in the endemic area seems to be entirely due to the process that leads to cretinism. There is no reason to describe deafness and deafmutism in an area with severe endemic goitre as a separate entity. The hearing defect showed a definite greater loss in the higher frequencies than in the lower frequencies and was found in 92% of the cretins. Deafmutism was present in 5, a loss of more than 60 db in 8, a loss of 40-60 db in 10 cretins. A loss of 20-30 db was found in 2.2% of normal subjects in the endemic area and 1.8% of those living in the control area. It is concluded that audiometry is a simple and significant test to establish the presence of the neurological form of endemic cretinism, which is the most prevalent form in most endemias. The differential diagnosis and pathogenesis of the described hearing defect are discussed.     

湖北省郧阳地区地方性甲状腺肿和地方性克汀病流行区环境微量元素的测定分析

本文报告了湖北省郧阳地区地甲病、地克病病区与非病区粮食、蔬菜和饮水中微量元素的测定分析结果。病区环境锌、铜含量显著低于非病区,这与病区人群头发分析结果相一致,表明病区人群锌、铜摄入不足。对微量元素与地甲病、地克病的关系作了初步探讨,认为锌、铜等微量元素在该病的发生上起着重要作用。     

硒,铜,锌与粘肿型地方性克汀病发病关系的研究

对青海和新疆粘肿型地克病高发区患者血浆硒、血清铜、锌、Ｔ４、Ｔ３及ＴＳＨ进行了研究。结果发现，青海病区患者血硒明显降低，而新疆病区实际上不存在缺硒问题；神经型和粘肿型患者血硒无明显差异，表明缺硒与粘肿型地克病发病关系不密切。缺硒病区患者Ｔ３水平明显低于正常对照。两病区患者血锌均显著降低。     

Epidemiology and clinical characteristics of endemic cretinism in Sicily

In this study we report the prevalence of endemic cretinism in the general population of two iodine deficient areas in Northeastern Sicily that were described more than 10 yr ago. In addition, the individual characteristics of endemic cretins are considered in order to define the typical expressions of this major iodine deficiency disorder in Sicily. Forty-three mental defectives were identified: 22 were living in an area with three bordering and closely connected municipalities within the province of Messina and with a population of 17,485 inhabitants (prevalence = 0.13%). Three out of the 22 were school-age children. The other community, in the province of Catania, was constituted by a unique and mostly agricultural hamlet, and showed a prevalence of cretinism that was 0.68% (21/3,100). Among the 43 mental defectives, 16 (37%) presented prominent neuromotor and neurosensorial disorders, including deafmutism and were euthyroid, thus conforming to the neurological type of cretinism; 13 (30%) were hypothyroid and exhibited stunted growth without significant neuromotor and neurosensorial impairment (myxedematous cretins). In the remaining 14 individuals (33%) neurological disorders were associated with stunted growth and clinical or biochemical hypothyroidism. These data indicate that also in Sicily endemic cretinism is a continuum of a variety of forms: among these the pure neurological and pure myxedematous forms represent the two extremes. Our observations also indicate that endemic cretinism still represents a major public health problem in Sicily. The finding of 3 endemic cretins younger than 13 yr suggests the persistence of this disorder even in the presence of improved economic, social and nutritional conditions.